OBJECTIVE: To report the clinical characteristics, diagnosis, treatment and prognosis of one patient with primary cutaneous CD8⁺ aggressive epidermotropic cytotoxic T-cell lymphoma (CD8⁺ PCAECTL), and to strengthen the understanding of this extremely rare type of lymphoma. METHODS: The clinical manifestations, diagnosis, treatment course, and prognosis of one patient with CD8⁺ PCAECTL admitted to our hospital were retrospectively analyzed. RESULTS: The patient is a 42-year-old female, with infiltrative skin rash on naso-facial and back as the main clinical manifestations. After pathological examination of the affected skin tissue, immunohistochemistry, molecular biology, and imaging, the diagnosis was confirmed as CD8⁺ PCAECTL, T3aN₀M₀ stage. Alternating chemotherapy with CHOP/HD-MTX (methotrexate, 6 g/m²) regimen was administered, and achieved complete remission (CR) after 4 cycles. After undergoing chemotherapy with DHAP regimen (cisplatin 100 mg/m², d 1 + cytarabine 2 g/m², q 12h, d 2 + dexamethasone 40 mg/d, d 1-4), the patient was mobilized for peripheral blood stem cells using recombinant human granulocyte colony-stimulating factor (G-CSF), and a sufficient number of CD34⁺ cells were successfully collected. Preconditioning was conducted with the BEAM regimen, followed by consolidation therapy with autologous hematopoietic stem cell transplantation (AHSCT). The patient remained in a disease-free survival state after 20 months of follow-up post-AHSCT. CONCLUSION CD8⁺ PCAECTL is extremely rare in clinical practice, with insidious onset and difficult early diagnosis. It is mainly characterized by the proliferation of epidermotropic CD8⁺ cytotoxic T cells and aggressive clinical course. At present, there is still no unified standard for the optimal treatment regimen, and the prognosis is very poor. Consolidation therapy with AHSCT after achieving remission through induction chemotherapy can improve the survival and prognosis of the CD8⁺ PCAECTL patients.
Humans ; Female ; Adult ; Antineoplastic Combined Chemotherapy Protocols/therapeutic use* ; Lymphoma, T-Cell, Cutaneous/diagnosis* ; Retrospective Studies ; Prognosis ; CD8-Positive T-Lymphocytes ; Skin Neoplasms/diagnosis* ; T-Lymphocytes, Cytotoxic

Mycosis fungoides (MF) is the predominant type of primary cutaneous lymphoma, accounting for at least 40%. It usually occurs in middle to late adulthood with the average age of diagnosis between 55 and 60 years. Folliculotropic MF is a variant that clinically presents with patches, plaques, and unusual hair loss within lesions. It predominantly involves the head and neck region. This usually presents with intense pruritus and commonly with secondary bacterial infection. Cutis verticis gyrata is a rare skin condition characterized by thickening and folding of the scalp, resulting in a furrowed or ridged appearance, and rarely associated with mycosis fungoides. A 46-year-old male, Filipino, presented with a 1-year history of generalized follicular papules, erythematous plaques, and subsequently presented with cutis verticis gyrata. Histopathological examination revealed findings consistent with folliculotropic MF. This was further confirmed by CD3, CD4, CD5 which revealed strongly positive immunohistochemical staining. To the best of our knowledge, there is currently only one published case of folliculotropic MF that presented with cutis verticis gyrata and only four cases of cutis verticis gyrata published locally.

Lymphomatoid Papulosis (LyP) is a rare skin disorder characterized by chronic, recurrent papules and nodules that heal spontaneously. This report discusses an 8-year-old Filipino female with a three-month history of erythematous pruritic papules evolving into plaques. A skin biopsy confirmed CD30-negative LyP, and treatment with Methotrexate resulted in significant improvement of the lesions. This case illustrates the complex diagnostic and therapeutic journey of LyP in children, emphasizing the importance of careful clinicopathologic correlation and the challenges of management due to the lack of curative therapy and the risk of malignant transformation.

Mycosis fungoides is the most common type of cutanoues T-cell Lymphoma. It is a mature T-cell non-Hodgkin lymphoma with presentation in the skin but with potential involvement of the nodes, blood, and viscera. This case reports a 24-year-old female who presented with a two-year history of progressively increasing hypopigmented macules and patches. Skin punch biopsy and immunohistochemical stains done were suggestive of a malignant T-cell process. The patient was managed with a short course topical corticosteroids and maintained on a topical moisturizer. The patient underwent multiple sessions of narrowband UV-B Phototherapy at 2-3 times per week and showed significant improvement of the skin lesions hence, was advised to be maintained on phototherapy.

Mycosis fungoides (MF) is a primary cutaneous T-cell lymphoma that may, although rarely, present as asymptomatic hypopigmented lesions. On the other hand, vitiligo is an autoimmune disorder characterized by asymptomatic depigmented patches. Few reports have documented both conditions occurring in a single patient, making this an intriguing diagnostic and management dilemma.

This is a case of a 26-year old female with a 10-year history of persistent hypopigmented, depigmented and erythematous patches and thin plaques [Figures 1a and 2a]. Histopathology showed epidermotropism of mononuclear cells on H&E stain [Figure 3] and a positive CD-3 stain for T cells, confirming mycosis fungoides [Figure 4b]. No evidence of lymphadenopathy and visceral involvement was found on both the chest and abdominal CT scans. The depigmented patches were likewise biopsied showing lack of Melan-A staining and absence of epidermal melanocytes, consistent with vitiligo [Figure 4a]. Final diagnoses were both mycosis fungoides, stage IB and vitiligo. The patient underwent narrowband UVB phototherapy and was started on topical corticosteroids [Figures 1b and 2b]. Improvement was evident over a three-month period, with body surface area decreasing from 77% to 46%. As the patient is young, has normal LDH levels, and shows no signs of extracutaneous disease, prognosis is promising.

This rare case of mycosis fungoides and vitiligo occurring together presents notable diagnostic and management challenges. Some studies hypothesize that the aberrant T cells involved in MF might contribute to the T cell-mediated destruction of melanocytes, similar to the mechanisms seen in vitiligo.

For diagnosis of rare conditions, consistent follow-up on the part of the patient as well as a high index of suspicion on the part of the physician is needed. Evaluation of the management should be done in the event that patients do not respond to treatment.

This is a case of a 33 year old female who fifteen months prior, noticed erythematous scaly plaques on her cheeks with mild pruritus. She was treated for psoriasis with Halobetasol ointment and Petroleum Jelly, which had partial resolution. Twelve months prior, she was prescribed Methotrexate, again achieving partial resolution. Biopsy was done suggestive of Folliculotropic Mycosis Fungoides. Methotrexate was increased, and additional medications were prescribed, leading to lesion resolution. However, she was lost to follow-up and experienced worsening symptoms. One month prior, biopsy was repeated and again showed Diffuse Lymphocytic Dermatitis positive for CD3+, CD4+, CD5+, CD8-, CD20-, CD30-, and loss of staining for Pan-T cell markers, CD2- and CD7. She is currently managed with regular sessions of Narrowband UVB.

These rare cases are few but more often than not, they are easily missed and when caught are usually progressed and already difficult to treat. Physicians must be vigilant in treating patients, even if they initially diagnose it to be a commonly seen and easily managed disease. Skin Directed therapy is done with PUVA and NBUVB with complete response in 30-70%3. For prognosis, early stages are favorable with a 94% 5 year survival rate, decreasing to 69% after tumor development.

Mycosis Fungoides is the most common type of primary cutaneous lymphoma. Early in its course, it usually presents as erythematous patches and plaques similar to a lot of cutaneous conditions. A 68-year-old male presented with a 13-year history of multiple erythematous patches and plaques on the arms and trunk. The lesions were pruritic and chronically relapsing over the years, temporarily relieved by topical corticosteroids. Thereafter, there was onset of multiple nodules on the trunk, extremities and face. Initial biopsy was done by a different dermatologist, revealed Hansen’s Disease, Borderline Borderline spectrum. Fitefaraco stain was positive but no bacillary index was given. The patient was subsequently started on rifamcpicin 600mg/capsule once a day, Ofloxacin 400mg/tablet once a day, and Minocycline 100mg/capsule once a day. The lesions were noted to worsen, eventually developing ulcerations over the trunk and extremities prompting referral to our institution. The biopsy results were as follows: Cutaneous T-Cell Lymphoma, diffuse cluster of differentiation (CD) 3 staining, focal CD20 staining, and negative FiteFaraco stain. The histopathologic findings combined with the clinical presentation led to the diagnosis of Mycosis Fungoides. He was then referred to medical oncology for proper staging and definitive management. The prognosisof Mycosis Fungoides is generally dependent on the stage as determined by the extent of skin involvement as well as presence of lymph node invasion and/or metastasis.
Mycosis Fungoides ; Lymphoma ; T-Cell ; Cutaneous

Primary cutaneous anaplastic large cell lymphoma is a rare non-Hodgkin's lymphoma.The tumor cells have the characteristics of anaplastic cells,expressing CD30 but not anaplastic lymphoma kinase.In this study,we reported a case of primary cutaneous anaplastic large cell lymphoma in a Tibetan child and summarized the clinicopathological features,aiming to strengthen the understanding of this disease.
Child ; Humans ; Ki-1 Antigen ; Lymphoma, Primary Cutaneous Anaplastic Large Cell ; Skin Neoplasms/pathology*

Humans ; Lymphoma, T-Cell, Cutaneous/complications* ; Neuroglia ; Pruritus/pathology* ; Skin Neoplasms/complications* ; Spinal Cord/pathology* ; Sympathetic Nervous System

Aminopyridines ; Benzamides ; Humans ; Programmed Cell Death 1 Receptor ; Sezary Syndrome/drug therapy* ; Skin Neoplasms/drug therapy*